Early and late results after surgical treatment of pulmonary atresia with intact ventricular septum]

Between 1970 und 1989 30 children were admitted with the diagnosis of pulmonary atresia with intact ventricular septum (PA/IVS). Before palliation 4 children died. According to the grade of right heart hypoplasia the patients were divided into 3 groups of mild, moderate or severe hypoplasia. Palliative operations were performed in 25 children (17 m, 9 f) with a mean age of 10 days: 13 valvotomies (V), 5 aortopulmonary shunts (S), 7 V plus S. One patient had total correction as primary procedure. A total of 17 reoperations was necessary in 12 of 26 patients (10 palliations, 7 total corrections). Total corrections were: 2 conduits and 5 patches of the right ventricular outflow tract (RVOT). Total mortality was 14/30 (54%) children: early 10/26 (38%), late 4/16 (25%) children. After total correction mortality was 3/7 (43%) children. Actuarial survival after palliation was 46% after 5 and 10 years. For patients with PA/IVS we recommend the following surgical strategy: 1. mild hypoplasia: V plus S for palliation; 2. moderate hypoplasia: S plus patch of RVOT; 3. severe hypoplasia: after initial ballon septostomy S and antegrade decompression of the right ventricle (RV). For total correction in a well developed RV we prefer ASD-closure and patch of RVOT if possible with homograft monocusp. In moderate or severe hypoplasia a Fontan operation is done with closure of the ASD and tricuspid orifice with a single patch.     

Postoperative course after first-stage palliation of hypoplastic left heart syndrome with right ventricle-to-pulmonary artery shunt; compared with classic Norwood procedure

BACKGROUND: A recent modification of the Norwood procedure involves the use of a right ventricle-to-pulmonary artery (RV-PA) shunt to provide pulmonary blood flow for patients with hypoplastic left heart syndrome (HLHS). We investigated the hemodynamics after first-stage palliation of HLHS with RV-PA shunt compared with classic Norwood procedure with subclavian-to-pulmonary artery (BT) shunt. METHODS: The postoperative course of 12 infants who had undergone first-stage palliation for HLHS using BT shunt (group BT: n=6) and RV-PA shunt (group RV-PA: n=6) were retrospectively reviewed and we obtained the following data: blood pressure, heart rate, inotropic support, atrial pressure, lactate, base excess, PaO2, FIO2. RESULTS: The RV-PA shunt using a non-valved conduit provided higher diastolic blood pressure than the BT shunt, but no significant difference in heart rate, systemic blood pressure, inotropic support and atrial pressure was observed between the two groups. Although the infants in the group RV-PA required significantly more myocardial ischemic time for operative procedure than those in the group BT, the serum lactate level in the group RV-PA was significantly lower than those in the group BT. CONCLUSIONS: These results show that the RV-PA shunt provides a stable systemic circulation and abundant tissue oxygen supply. Excellent hemodynamics provided by RV-PA shunt is beneficial for infants undergoing stage I palliation for HLHS.     

Individualized surgical management of complete atrioventricular canal

In a 5 year interval 46 children with complete atrioventricular canal (CAVC) required 51 operations. Thirty-two children underwent correction; nine received surgical palliation. The ages of the children undergoing correction ranged from 4 months to 14.6 years (mean 4.2 years); eight were less than 1 year old. Weights ranged from 4.1 kg to 39 kg (mean 13.5 kg); 15 weighted less than 10 kg. Ten had undergone previous palliation (seven by pulmonary artery band; three by shunt). There were no early deaths and two late deaths. One infant required mitral replacement at correction; two required subsequent mitral replacement. The ages of the children undergoing palliation ranged from 8 days to 1.34 years (mean 5.8 months); 16 were less than 1 year old. Weights ranged from 2.5 kg to 8.5 kg (mean 4.4 kg); 14 weighted less than 5 kg. Operations included pulmonary artery banding in 14, shunt creation in four, and pericardial enlargement of the right ventricular outflow tract in one. One death occurred 5 days after pulmonary artery banding in an infant with unrecognized coarctation. One late death occurred several months after the creation of a second shunt in a child with severe tetralogy of Fallot and hypoplastic pulmonary arteries. Forty-two (91%) of these children were alive at the time of this review. The outcome in these 46 patients supports individualized choice of initial operation (palliation versus correction) based upon clinical condition, weight, and associated anomalies. The pulmonary artery is banded in infants less than 4 to 5 kg; larger infants and children undergo correction primarily.     

The Blalock-Taussig shunt for simple complete transposition of the great arteries followed by later anatomic correction. Report of 2 cases with conflicting postoperative courses

The Blalock-Taussig (BT) shunt was performed in two infants with simple transposition of the great arteries (TGA) after poor response to septostomy not only to improve hypoxia but also in expectation of preparing the left ventricle for later anatomic correction. One patient had adequate muscle mass and pressure of the left ventricle after the shunt and underwent successful anatomic correction. The other patient developed intractable heart failure following the shunt. Anatomic correction was performed with success in this patient after pulmonary artery banding and takedown of the BT shunt. Based on these experiences, if heart failure does not develop or is controllable after BT shunt, there may be a possibility of later anatomic correction. However, if uncontrollable failure occurs, the shunt should be taken down and arterial or venous switch correction should be performed as soon as possible.     

The indication and effects of palliative right ventricular outflow tract reconstruction for tetralogy of Fallot with pulmonary atresia]

Palliative right ventricular outflow reconstruction (RVOT) in 5 cases with tetralogy of Fallot with valvular pulmonary atresia and 5 cases with truncal pulmonary atresia were studied regarding indication and postoperative development of pulmonary artery. In 4 cases with valvular atresia, RVOT was performed following previous palliation such as Blalock-Taussig shunt, and resulted in improvement in clinical symptoms and in significant increase in postoperative PA-index from 105.4 mm2/m2 before operation to 159.4 mm2/m2. In one case, RVOT was performed as the first stage palliation and symmetrical development of pulmonary arteries were observed. All underwent successful total correction later. However, in 5 cases with the truncal atresia, 2 died after RVOT and postoperative development of pulmonary artery was insignificant. RVOT is indicated for cases with poor pulmonary arterial development despite of palliation, and in future, RVOT as an initial palliation will be indicated in infants with hypoplastic pulmonary artery.     

The Blalock-Taussig Operation: The Procedure of Choice in the Hypoxic Infant with Tetralogy of Fallot

From 1971 to 1975, 17 consecutive patients aged 1 day to 4 years underwent Blalock-Taussig shunts for severe tetralogy of Fallot. Three infants were under 6 weeks of age and 7 (41%) under 1 year. There were no hospital deaths. Modification of the shunt technique adapts it to any size infant. The subclavian artery is divided at its major branches and the end spatulated to enlarge it. The artery is occluded while the shunt is constructed.No intraoperative complications were encountered; all patients have a shunt murmur with no early or late closure. No child has had heart failure or hypoxic spells. Flows measured at operation equaled one-quarter to one-half of the child's normal cardiac output. Ligation of the shunt at subsequent repair is uncomplicated. One child died three years later at correction from causes unrelated to the shunt.With appropriate modifications in technique, the Blalock-Taussig shunt is the operation of choice, at any age, for palliation of severe tetralogy of Fallot.     

Modified Blalock-Taussig shunts: results in infants less than 3 months of age.

The optimal procedure for shunting palliation in cyanotic infants remains to be determined. Sixty-two infants less than 3 months of age underwent 63 modified Blalock-Taussig shunts. Their age range at operation was 1 to 84 days (mean, 16 +/- 20 days). Shunts were constructed using 5-mm polytetrafluorethylene tubes in 20 patients and 4-mm polytetrafluoroethylene grafts in 43 patients. There were 13 early deaths (21%; CL, 15% to 27%) of which three deaths (5%; confidence limits, 2% to 9%) were shunt related. The survivors were followed up from 6 to 53 months (mean, 29 +/- 12.5 months). Shunt failure (occlusion, inadequate palliation) occurred in 27 patients. The overall probability rate of adequate shunt function was 58% +/- 8% at 2 years. Univariate and multivariate analyses showed that the size of the graft was a risk factor of shunt failure. Severe distortion of the pulmonary arterial branch was noted in 12 patients. The inferences are: (1) modified Blalock-Taussig shunts provide satisfactory early palliation but late shunt failure is frequent; (2) similar results should be obtained with other shunting procedures; and (3) the optimal procedure should be selected for each cyanotic infant on an individual basis.     

Total correction of transposition of the great arteries in infancy as initial surgical management

Fifteen patients have undergone total correction of transposition of the great arteries (TGA) in the past two years. Four were under 6 months of age and weighed 3.4, 3.5, 4.3, and 5.7 kg., respectively; 6 were from 13 to 21 months old; and 5 were 2 to 10 years old. In this series, balloon atrio-septostomy (BAS) provided palliation that was brief and often unsatisfactory. All 8 patients who had BAS without surgical septectomy had severe cyanosis, exercise intolerance, and retardation of growth and development prior to correction. There were 2 operative deaths related to complicated TGA and 1 unrelated late death. All survivors have improved dramatically. Survival alone is therefore no longer considered to be successful palliation following BAS. If adequate relief of cyanosis and reasonable growth and development are not achieved, total correction in infancy is a favorable alternative to surgical septectomy.     

Systemic to pulmonary arterial shunts in neonates

Background The major strategy for palliation of cyanotic lesions in neonates is the systemic to pulmonary arterial shunt. Methods Between May 1995, and December 2002, 48 consecutive neonates underwent systemic to pulmonary arterial shunts for cyanosis with reduced pulmonary blood flow. The mean age was 11.6 days (±SD 7.38) and the mean weight, 3.2kg (±SD 0.52). The babies were classified into three groups: Group I-Tetralogy-pulmonary Atresia (n=18), Group II-single Ventricle-Pulmonary atresia without (n=19) and with (n=5) isomerism, Group III-Pulmonary Atresia with Intact ventricular septum (n=6). Diagnosis was made by 2D echocardiography. Indication for cardiac catheterization was delineation of pulmonary anatomy/ductus laterality (n=4) or balloon atrial septostomy (n=4). The surgical procedure was a modified Blalock-Taussig shunt on the side of the situs. Post-operatively, no anti-coagulation or anti-platelet medication was employed. Results There was no mortality. Four cases required revision of the shunt in the immediate post-operative period for shunt thrombosis. The mean follow up was 17.54 months (±SD 8.36). In Group I, nine patients have undergone total correction with or without a conduit, while three required new arterial shunts for shunt/pulmonary artery stenosis. In Group II, nine patients have undergone bi-directional Glenn with atrial septectomy (n=2) and pulmonary artery plasty (n=4) and one patient underwent Fontan completion. In Group III, two patients underwent bi-directional Glenn and two had pulmonary valvotomy with/without right ventricular outflow tract widening. All the remaining babies are waiting for the second/final stage palliation or total correction. Conclusion Systemic to pulmonary arterial shunts in neonates is a gratifying and reasonably safe surgical procedure. Most babies become candidates for eventual univentricular/bi-ventricular repair.     

Thirty-year follow-up of superior vena cava-pulmonary artery (Glenn) shunts.

The first superior vena cava-pulmonary artery shunt (Glenn shunt) in our series was performed in February 1958. From then through September 1988, 91 patients have undergone this procedure for a wide variety of congenital defects. We here report follow-up data available on all patients. Ages ranged from 2 days to 46 years (mean 6.8). Diagnoses were as follows: tricuspid atresia, 27; single ventricle, 22; tetralogy of Fallot, 14; D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis, 9; D-transposition, 5; Ebstein's anomaly, 4; pulmonary atresia + intact septum, 4; and others, 6. The hospital mortality rate was 7.7% (one death in the last 53 patients, 1.9%). Five deaths occurred in patients less than 6 months old. There were 20 late deaths (22%) with actuarial survival rates of 84% and 66% at 10 and 20 years, respectively. Pulmonary arteriovenous fistula formation was seen in 18 patients (19.7%), six of whom have undergone therapeutic embolization with improvement in saturation. The prevalence of pulmonary arteriovenous fistula increases with time after shunt. No long-term shunt thrombosis or stricture formation was seen. Fifty percent of shunts were still functioning at 20 years. Palliation was limited because of decrease in blood flow to the contralateral pulmonary artery, collaterals between the inferior and superior venae cavae, and pulmonary arteriovenous fistula formation. Improvement in saturation was obtained in eight otherwise inoperable patients by creation of a right axillary arteriovenous fistula up to 19 years after the Glenn shunt. Three patients had conversion of a Blalock-Taussig shunt to a Glenn shunt with improvement in congestive heart failure. Twenty-six patients have undergone a Fontan procedure with two deaths. Compared with the group having a Fontan procedure without a prior Glenn operation, there was no difference in early or late mortality. Thirty years after a Glenn shunt, the first patient in this series is working full time after having undergone a modified Fontan procedure in 1981. We conclude that the Glenn connection, usually with supplemental procedures to enhance oxygenation, has provided excellent physiologic palliation with low mortality up to 30 years with no late thrombosis or stricture formation. The incidence of pulmonary arteriovenous fistula increases with time and can be effectively treated with embolization. Physiologic repair after the Glenn shunt carries a low mortality. Although currently used infrequently, superior vena cava-pulmonary artery shunting remains a useful method of palliation in selected patients.(ABSTRACT TRUNCATED AT 400 WORDS)     

经右心室流出道肺动脉瓣球囊成形治疗室间隔完整型肺动脉闭锁的疗效分析

目的分析经右心室流出道肺动脉瓣球囊成形术(surgical balloon valvuloplasty,SBV)进行右心室减压联合个体化分流手术治疗室间隔完整型肺动脉闭锁(pulmonary atresia with intact ventricular septum,PA/IVS)的中远期疗效。方法回顾性分析2005年1月至2017年12月在本中心接受SBV治疗的91例PA/IVS患者的临床资料,其中男52例(57.1%)、女39例(42.9%),中位年龄3个月(1 d,24个月),中位体重4.1(2.5,12.0)kg。结果所有患者均行SBV,其中62例患者同期行其他外科手术,包括动脉导管未闭(patent ductus arteriosus,PDA)结扎术33例;PDA结扎术+改良Blalock-Taussig分流术23例;PDA结扎术+双向格林手术6例。没有早期死亡。中位随访时间8.8(2.5,13.4)年,4例(4.4%)失访。随访患者中,7例(8.0%)患者死亡,1例(1.1%)患者因肺动脉瓣狭窄再次行SBV。在随访中,5例(5.7%)行一个半心室矫治术,2例(2.3%)行Fontan手术;平均三尖瓣Z值为?1.7±1.5,较术前明显增加(t=5.587,P<0.001)。结论通过SBV联合个体化分流手术策略治疗PA/IVS是安全、有效的,大多数患儿可达到双心室矫治,避免单心室姑息治疗。     

Bi-directional cavopulmonary shunt: is accessory pulsatile flow, good or bad?

OBJECTIVE: Evaluation of the effect and long-term outcome of accessory pulsatile blood flow versus classical bi-directional cavopulmonary connection (BCPC). METHODS: Retrospective review of the medical and surgical records. RESULTS: Two-hundred and five patients (119 boys, 86 girls) underwent BCPC from 1990 to 1996. Accessory pulsatile flow was present in 68%, flow being maintained through the pulmonary trunc in 46%, systemic-to-pulmonary artery shunt in 13% and mixed in 7%, or patent ductus arteriosus in 2%. Patients with accessory pulsatile flow had lower hospital mortality (3% versus 5%), while mean pulmonary artery pressure (14.1 versus 12.6 mmHg P = 0.050) and increase of oxygen saturation (12.4 versus 8.7, P = 0.034) were significantly higher. The period of artificial ventilation (1.9 day) and ICU stay (6 days) did not differ for both groups. Late mortality was higher following accessory pulsatile flow (6% versus 1%). At late follow-up patients with accessory pulsatile flow had significantly higher oxygen saturation (mean 85 +/- 4%, versus 79 +/- 4%; P < or = 0.005). If subsequent completion of Fontan is considered the optimal palliation and subsequent systemic to pulmonary artery shunt, arteriovenous fistula and transplantation is considered a failure, patients with accessory pulsatile flow had significantly more and earlier completion of the Fontan procedure (mean 1.7 +/- 2.4 years, versus 2.7 +/- 4.4 years; P = 0.008). Survival is not influenced by age at bi-directional cavopulmonary shunt surgery, left or right functional ventricular anatomy or previous palliative surgery. One patient with accessory pulsatile flow developed systemic-to-pulmonary collateral's eventually requiring lobectomy. CONCLUSION: Despite two different initial palliative techniques the outcome was not significantly different. Accessory pulsatile blood flow appeared not to be a contra-indication for a completion Fontan procedure. Moreover, the data suggest that after accessory pulsatile flow can safely be performed, at late follow-up oxygen saturation is higher, while, significantly more and earlier completion of Fontan occurred. Age at bi-directional cavopulmonary shunt, basic left or right ventricular anatomy or previous palliative surgery did not influence survival.     

Pulmonary atresia with intact ventricular septum. Sixteen-year experience.

Infants with pulmonary atresia and intact ventricular septum (PA-IVS) usually require urgent surgical intervention. Thirty patients with this anomaly, seen at the Columbia-Presbyterian Medical Center between 1962 and 1978, had palliative operations, 26 within the first 3 days of life. Six underwent a closed pulmonary valvotomy alone, with no survivors; six had only a systemic--pulmonary artery shunt, with three early survivors. Because of this experience, 17 had a combined procedure of valvotomy and shunt, with 14 early survivors. One patient recently underwent a definitive right ventricular outflow patch procedure with cardiopulmonary bypass. Eight patients subsequently have had corrective open-heart procedures, with five patients surviving from 2 to 10 years. A unicusp aortic homograft was used for repair in five and a Hancock valved conduit in three. Four patients are presently awaiting operation. We conclude that the initial surgical management of these critically ill infants must not only increase pulmonary blood flow but in addition provide an opportunity for right ventricular growth. Thus we continue to advocate the combined procedure of a valvotomy plus a shunt to provide adequate palliation. Repeat catheterization should be performed within a year to confirm the adequacy of the valvotomy, since this is essential to maximal right ventricular enlargement and to allow for definitive correction at a later date.     

Bi-directional glenn shunt under veno-venous bypass—A reality

Background Bi-directional Glenn shunt as first surgery or palliation for patients with Single Ventricle physiology is usually done with cardiopulmonary bypass. We have studied the feasibility of doing this procedure under venovenous bypass. Material & Methods Out of 45 patients undergoing univentricular heart repair, 14 patients (TA, VSD, PS: 9; DORV, VSD, PS: 2; SV, PS: 2; DTGA, VSD, PS: 1) underwent Bi-directional Glenn procedure under venovenous bypass between October 2002 to July 2003. The outcomes of this procedure were analyzed in terms of post-operative ventilatory support, improvement in oxygen saturation, and echo documentation of shunt flow and hospital stay. Results The mean superior vena caval pressure during veno-venous bypass was 24mmHg, which dropped to 16mmHg in the intensive care unit. The oxygen saturation improved from 65% (mean), to 88% (mean) post operatively. The average post-operative ventilatory support was 14.6 hrs and intensive care stay was 2.3days (mean). There was no neurological deficit noticed in any patient nor any patient had prolonged pleural effusion. There was one death in the study group due to progressive myocardial failure. Conclusion Bi-directional Glenn procedure under veno-venous bypass is safe, and can be done with favorable post-operative out come comparable to conventional way at much less expenditure, preventing exposure of the patients to the ill effects of cardiopulmonary bypass.     

The role of cava-pulmonary (Glenn) anastomosis in the palliative treatment of congenital heart disease

The value of the cava-pulmonary anastomosis (Glenn procedure) was assessed by reviewing the entire experience with this procedure in 83 children at The Hospital for Sick Children, Toronto, from 1961 to 1980. The over-all operative mortality rate was 9.6%, but only 2% in the second half of the series. The average follow-up is 9.4 years per patient. The late mortality rate has been 20%. By actuarial analysis, 84% of survivors were alive 9 years after operation; 54% had adequate palliation and 46% had required further operation. Long-term palliation was significantly better when the anastomosis was performed after 5 years of age. A previous arterial pulmonary artery shunt had no effect on either patient or shunt survival. Patients with tricuspid atresia had a better long-term survival rate than, but similar shunt survival to, those children with transposition, single ventricle, or miscellaneous cardiac anomalies. Angiography in 36 survivors showed patent shunts in 35 and no arteriovenous fistulas. Clinical status was not related to superior vena caval pressure or angiographic findings, indicating that Glenn shunt failure is a functional problem. The procedure is most useful for patients over 5 years of age who have complex heart disease not amenable to total repair. It is also on appropriate initial procedure in young patients over 18 months of age for whom direct atrium--pulmonary artery anastomosis is proposed as the future definitive treatment.     

The Blalock-Hanlon operation: an anachronism?

Some surgeons advocate early correction of dextrotransposition of the great arteries (D-TGA) using hypothermic circulatory arrest when Rashkind balloon atrial septostomy is inadequate. An alternative procedure is a Blalock-Hanlon closed atrial septectomy. From 1971 to 1985, 19 patients underwent the latter procedure for D-TGA at our institution. Three had it as the first procedure, and 16 had undergone Rashkind balloon atrial septostomy, which was unsuccessful. Age at performance of the Blalock-Hanlon closed atrial septectomy ranged from 1 day to 5 months (mean, 2.51 months). There were no operative deaths. Subsequently, 18 children have had a Mustard procedure, and 1, a Rastelli procedure (average age at definitive repair, 25.31 months). One death followed definitive repair (combined mortality, 5%). On room air, average partial pressure of arterial oxygen rose from 23.25 mm Hg prior to closed atrial septectomy to 31.45 mm Hg at discharge. Average hematocrit fell from 58.37% prior to closed atrial septectomy to 53.94% prior to definitive repair. At the time of definitive repair, percentile height ranged from the 3rd to the 95th percentile (mean, 44th percentile) and weight ranged from the 3rd to the 90th percentile (mean, 32.5th percentile). In 1 infant, transient neurological symptoms developed three days following Blalock-Hanlon atrial septectomy. The symptoms were due to multiple cerebral infarctions thought to be caused by emboli. No permanent neurological deficit resulted. We believe that the infant with an inadequate Rashkind balloon atrial septostomy prior to age 6 months should undergo a Blalock-Hanlon septectomy followed by definitive repair timed on the basis of the baby's growth, associated anomalies, and evidence of pulmonary hypertension.     

室间隔完整型肺动脉瓣闭锁介入治疗与外科手术的临床对比

目的 比较经导管介入治疗与外科手术的室间隔完整型肺动脉瓣闭锁(pulmonary valveatresia with intact ventricular septum,PA/IVS)临床治疗效果,探讨PA/IVS安全、有效的首期治疗方法 .方法 2006年1月至2009年5月,收治25例PA/IVS病儿中男20例,女5例.首期治疗年龄2天～8个月.体重2.1～6.7 kg.超声心动图提示右室三部分存在,右室轻～中度发育不良(三尖瓣Z值在-2～1.5),无右室依赖型冠状循环.经导管介入治疗(介入组)8例,外科手术(手术组)17例.结果 两组成功率分别为88%和94%,两组各死亡1例.两组术后残余肺动脉瓣狭窄(PS)程度无明显差异,介入组机械通气时间及术后住院时间较之手术组短.全组随访3～36个月,介入组术后1例再次行球囊扩张,手术组术后行PS外科矫治和PS球囊扩张各1例,2例等待球囊扩张术.全组21例获双心室循环,介入组与手术组各1例因右心功能不全拟行双向格林手术.结论 对于右室轻～中度发育不良的PA/IVS,经导管介入治疗可以取代外科手术作为首期治疗方法 .     

室间隔完整型肺动脉瓣闭锁介入治疗与外科手术的临床对比

目的 比较经导管介入治疗与外科手术的室间隔完整型肺动脉瓣闭锁(pulmonary valveatresia with intact ventricular septum,PA/IVS)临床治疗效果,探讨PA/IVS安全、有效的首期治疗方法 .方法 2006年1月至2009年5月,收治25例PA/IVS病儿中男20例,女5例.首期治疗年龄2天～8个月.体重2.1～6.7 kg.超声心动图提示右室三部分存在,右室轻～中度发育不良(三尖瓣Z值在-2～1.5),无右室依赖型冠状循环.经导管介入治疗(介入组)8例,外科手术(手术组)17例.结果 两组成功率分别为88%和94%,两组各死亡1例.两组术后残余肺动脉瓣狭窄(PS)程度无明显差异,介入组机械通气时间及术后住院时间较之手术组短.全组随访3～36个月,介入组术后1例再次行球囊扩张,手术组术后行PS外科矫治和PS球囊扩张各1例,2例等待球囊扩张术.全组21例获双心室循环,介入组与手术组各1例因右心功能不全拟行双向格林手术.结论 对于右室轻～中度发育不良的PA/IVS,经导管介入治疗可以取代外科手术作为首期治疗方法 .     

室间隔完整型肺动脉瓣闭锁介入治疗与外科手术的临床对比

目的 比较经导管介入治疗与外科手术的室间隔完整型肺动脉瓣闭锁(pulmonary valveatresia with intact ventricular septum,PA/IVS)临床治疗效果,探讨PA/IVS安全、有效的首期治疗方法 .方法 2006年1月至2009年5月,收治25例PA/IVS病儿中男20例,女5例.首期治疗年龄2天～8个月.体重2.1～6.7 kg.超声心动图提示右室三部分存在,右室轻～中度发育不良(三尖瓣Z值在-2～1.5),无右室依赖型冠状循环.经导管介入治疗(介入组)8例,外科手术(手术组)17例.结果 两组成功率分别为88%和94%,两组各死亡1例.两组术后残余肺动脉瓣狭窄(PS)程度无明显差异,介入组机械通气时间及术后住院时间较之手术组短.全组随访3～36个月,介入组术后1例再次行球囊扩张,手术组术后行PS外科矫治和PS球囊扩张各1例,2例等待球囊扩张术.全组21例获双心室循环,介入组与手术组各1例因右心功能不全拟行双向格林手术.结论 对于右室轻～中度发育不良的PA/IVS,经导管介入治疗可以取代外科手术作为首期治疗方法 .     

Follow-up study of pulmonary artery configuration in hypoplastic left heart syndrome

Objective. Pulmonary artery (PA) distortion significantly compromises the outcome of the staged approach to the Fontan operation in patients with hypoplastic left heart syndrome (HLHS). This retrospective study was designed to investigate the influence of the initial operation on postoperative PA anatomy. Methods. Forty-nine patients with HLHS and its variant were enrolled in this study. As an initial palliation, the Norwood operation with a modified Blalock-Taussig (BT) shunt was performed in 12, the Norwood operation with a right ventricle to pulmonary artery (RV-PA) shunt in 31, and bilateral PA banding in 6. The incidence and risk factors of postoperative central pulmonary artery stenosis (PS) were investigated, and the PA configuration was followed up until post-Fontan status. Results. Twenty-two patients (51.2%) had developed central PS after the Norwood operation (33.3% with a BT shunt vs. 58.1% with a RV-PA shunt). The RV-PA shunt with a polytetrafluoroethylene (PTFE) patch at the distal pulmonary stump significantly decreased the central PS (P = 0.035). The PA index after the Norwood operation was not statistically different between the BT and RV-PA shunt groups, although in the RV-PA group it was significantly higher in patients with a PTFE patch on the distal PA stump. PA plasty was performed in 16 patients in the second-stage palliation and in 15 with the Fontan completion. Freedom from PA plasty was significantly lower in the RV-PA shunt group than in the BT shunt group (63.5% vs. 31.1% at 5 years, P = 0.034). Six patients initially palliated with bilateral PA banding had no stenosis at the banding site in the Norwood + Glenn operation, and one patient required stent placement for left PS in the Fontan completion. Post-Fontan catheterization (n = 31) showed central venous pressure of 11.5 ± 2.6 mmHg, cardiac index of 3.6 ± 0.8 l/kg/min, and PA index of 194.0 ± 58.4 mm²/m²; there was no difference between the groups. Conclusion. The incidence of central PS after the Norwood operation was significant, and the shunt type and procedure for the distal PA stump influenced the postoperative configuration of the central PA. With an aggressive surgical approach to central PS, PA anatomy was satisfactory with good hemodynamic variables after Fontan completion. Bilateral PA banding did not cause later vascular deformity. Presented at the 59th Annual Scientific Meeting of the Japanese Association for Thoracic Surgery, held in Tokyo, Japan, October 1–4, 2006